Introduction: Ewing's sarcoma (ES) is an extremely rare bone malignancy observed in otorhinolaryngeal practice. In otorhinolaryngology, it sometimes involves the facial bones and cervical vertebrae. In children, ES is the second most common primary malignancy of bone after osteosarcoma. Extraskeletal Ewing’s sarcoma (EES) is an extremely rare malignancy of mesenchymal cell origin. The EES is observed in the trunk and lower extremities. The neck is an extremely rare primary site for EES.

Case Report: A 3-year-old female child referred with a rapidly enlarging right-sided neck swelling since 2 months ago. Physical examination and preoperative investigations suggested the diagnosis of a carotid body tumor and histopathological findings were similar. However, the results of immunohistochemistry revealed a confirmatory diagnosis of ES. Conclusion: Hereby, we presented a case of EES of the neck in a child, which was completely misdiagnosed preoperatively due to its rare incidence. The incidence of EES in the head and neck region can be successfully managed with radical excision and radiotherapy.